Pemphigus vulgaris

This code represents pemphigus vulgaris, a rare, chronic autoimmune blistering disease affecting the skin and mucous membranes. It is characterized by the formation of flaccid bullae and erosions due to autoantibodies targeting desmoglein 1 and 3, leading to acantholysis.

Use this code for patients diagnosed with pemphigus vulgaris, confirmed by clinical presentation, biopsy showing acantholysis, and direct immunofluorescence demonstrating intercellular IgG and/or C3 deposition in the epidermis. This code is appropriate for initial diagnosis and subsequent encounters for management of the condition.

• Ensure documentation clearly specifies "vulgaris" to differentiate from other pemphigus subtypes.
• Consider additional codes for complications such as secondary infections (e.g., bacterial, fungal) of the bullae or erosions.
• Review pathology reports and immunofluorescence findings to support the definitive diagnosis.